PB2574: HEMOPHAGOCYTIC SYNDROME IN ADULTS - REAL-WORLD DATA ON MORTALITY FROM A TERTIARY REFERENCE CENTER
Authors: Tatiana Wojtovicova, Drahomir Aujesky, Stephan Jakob, Nicolas Bonadies, Hansjakob Furrer, Urban Novak, Thomas Pabst, Britta Maurer, Burkhard Möller, Roman Christian Abegglen, Aristomenis Exadaktylos, Annalisa Berzigotti, Yara Banz, Vera Ulrike Bacher, Sacha S. Zeerleder, Joerg C. Schefold, André Tichelli, Anne Angelillo-Scherrer, Alicia Rovó
Journal: HemaSphere (2023)

Topic: 30. Infections in hematology (incl. supportive care/therapy) Background: Hemophagocytic Syndrome (HLH) is a rare, life-threatening condition characterized by hyperinflammatory response associated with aberrant macrophages activation and cytokines storm. Real-world data on HLH in adults are sparse. Aims: In this study, we aimed to assess the clinical characteristics and outcomes, particularly mortality, of HLH adult patients in our tertiary referral hospital. Methods: The hospital database (Microsoft SQL Server management Studio) was searched to identify patients ≥18 years of age who were diagnosed as HLH between January 2014 and June 2021. Using a stepwise process, we first search for patients in which HLH diagnosis was mentioned in the medical record. Subsequently, we used the Saint Antoine score (HScore) (L. Fardet et al. Arthritis & Rheumatology 2014), to evaluate the data. Overall survival (OS) was estimated by Kaplan-Meier method. A logistic regression analysis to predict death included all variables with p value <0.10 at univariate analysis. A forward stepwise model excluded parameters that were no longer significant. Results: The search was conducted across available medical reports of 845,846 adult patients. Overall, the diagnosis of HLH was mentioned in 79 patients. After the exclusion of 24 duplications, the remaining 55 patients were examined using the HScore of whom 54 patients meet the eligibility criteria and were included in the analysis (0.006% of all adult patients). Analyzed data are summarized in Table 1. The median age at HLH diagnosis was 61 years (r 22 – 83), the majority of patients were females (N=36, 66.7%). Five patients have a least one relapse during follow-up. The most frequent observed diagnostic criteria were cytopenia (98.1%), hyperferritinemia (92.6%), hypertriglyceridemia (85.2%) and fever (81.5%). In 37/54 (68.51%) patients at least one possible underlying cause was identified, in 4/54 (7.4%) more than one. Most frequent underlying identified causes were hematological neoplasms in 18/54 (33.3%), infections in 14/37 (25.92%), rheumatic disorder in 7/54(12.96%), 1 patient after CAR-T cell therapy and 31% were idiopathic. The OS at 180 days was 58%+±6.85, all but one death occurred in the first 30 days after diagnosis. When the clinical and laboratory characteristics were compared between the survivors and the patients who died in univariate analysis, the statistically significant unfavorable predictive factors were: neurological symptoms, cardiovascular complications, requiring platelet transfusion, increased alkaline phosphatase and age >50 years. In multivariate analysis, factors associated with relative risk of death after the diagnosis of HLH were older age at diagnosis (Relative Risk [RR] 1.175, 95%CI 1.0038-1.329; p=0.01); presence of cardiopulmonary complications (RR 254.9 (4.649-13979; p=0.007) and increased triglycerides at diagnosis (RR 465 (3.466-62572; p=0.014). Requiring platelets transfusions was borderline: RR 31.011 (0.799-1203; p=0.066). Summary/Conclusion: These data confirm the rare occurrence and high risk of dying from HLH in adults patients. The number of diagnostic criteria and a high HScore were not related with higher death rates. The main associated factors with overall mortality were advanced age, the presence of cardiovascular complications, and high triglycerides at diagnosis. Further awareness on this entity and multidisciplinary work are essential to improve outcome.Keywords: Hemophagocytic Lymphohistiocytosis (HLH), Inflammation